The tangled truth about Uncombable Hair Syndrome

The rare disease, which has produced less than 100 cases in medical journals since 1973, usually presents itself between the ages of 3 months and 12 years and is exactly as it sounds. The hair, which grows in silvery-blond or straw-colored, stands out straight from the scalp and is impossible to comb.

The problem, according to dermatologist Dr. David Orentreich of New York's Orentreich Medical Group, lies within the hair shaft.

"If you look at the hair close up, you'll see one or more channels or grooves running down the shaft," says Orentreich, assistant clinical professor at Mt. Sinai School of Medicine. "That imparts a different behavior to the hair. Normally, hair is quite pliable. You can run your fingers through it; it will bend easily when you comb it. But this makes the hair very difficult to comb. It just won't bend."

Affected hair is dry, curly, brittle, and progressively uncombable, eventually taking on a "spangled" or shimmery appearance, most likely due to the reflection of light off the irregular surface of the shaft. That shimmering may explain its more common name, "spun glass hair."

While only scalp hair is affected — there's no such thing as uncombable eyebrow syndrome — UHS is sometimes seen as part of a larger group of genetic abnormalities known as ectodermal dysplasia, a group of about 150 heritable disorders that affect the skin, hair, teeth and nails, etc.

UHS was first described in 1973 in a French medical study, which dubbed it "cheveux incoiffables." It has been found to be both genetic and sporadic. In a 1982 German study, uncombable hair syndrome was present in six members of one family. A 2007 medical journal reported the case of a family affected by the condition for four generations (some members also showed signs of abnormalities of the nails as well).

While the syndrome has only been recently scientifically recognized, it has been part of German literature for more than 150 years, Orentreich says. The popular children's fairy tale "Der Struwwelpeter" (Slovenly or Shaggy Peter) is a morality tale written by a Frankfurt physician in 1845 about a naughty little boy who refuses to groom himself properly. A Victorian illustration for the story shows a boy with unruly blonde hair and incredibly long nails. In 2000 the fairy tale was turned into the macabre opera, "Shockheaded Peter," which takes the children's misbehavior to a new dark level (the kids all die).

"The original story was probably based on children who had ectodermal dysplasia," says Orentreich. "These kids were observed back then with a much greater degree of ignorance with regard to medicine and health. If their hair was wild, people thought the kid was also wild."

Today, uncombable hair can sometimes be tamed with the use of biotin, also known as vitamin H, at least with regard to the hair's appearance (the strange structure of the hair is not affected). Other relief for tangling and uncombability may be found through the use of lubricating hair products which contain biotin or other moisturizers, says Osrentreich.

As for those of you with just plain bad hair, a German study (perhaps inspired by bedtime readings of Slovenly Peter) released last month has shed light on how hair fibers interact. Using an atomic force microscope and samples of Caucasian female hair, researchers at the University of Bayreuth in Germany found that damage to hair causes scaly projections to protrude from hair fibers. These projections create friction and make hair feel rough to the touch and hard to comb.

Not to be confused with rats' nests (which mothers discovered centuries ago), these insights into molecularly misbehaving hair may help researchers develop better hair products in the future.

As for uncombable hair, as one study puts it, "the hair is grossly abnormal in infancy and early childhood, but may have improved manageability later in life."

In other words, it will probably drive your poor mom crazy for a few years (and elicit a few unkind comments from insensitive strangers), but eventually, like so many other childhood peccadilloes - i.e., nose picking, scab eating and temper tantrums - it will simply disappear.